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QUESTION 1 You have a hybrid Exchange Server 2016 organization. Some of the mailboxes in the research department are hosted on-premises. Other mailboxes in the research department are stored in Microsoft Office 365. You need to search the mailboxes in the research department for email messages that contain a specific keyword in the message body. What should you do? A. From the Exchange Online Exchange admin center, search the delivery reports. B. Form the on-premises Exchange center, search the delivery reports. C. From the Exchange Online Exchange admin SY0-401 exam center, create a new In-Place eDiscovery & Hold. D. From the Office 365 Compliance Center, create a new Compliance Search. E. From the on-premises Exchange admin center, create a new In-Place eDiscovery & Hold. Correct Answer: E QUESTION 2 You have an Exchange Server 2016 organization. You plan to enable Federated Sharing. You need to create a DNS record to store the Application Identifier (AppID) of the domain for the federated trust. Which type of record should you create? A. A B. CNAME C. SRV D. TXT Correct Answer: D QUESTION 3 Your company has an Exchange Server 2016 200-310 exam Organization. The organization has a four- node database availability group (DAG) that spans two data centers. Each data center is configured as a separate Active Directory site. The data centers connect to each other by using a high-speed WAN link. Each data center connects directly to the Internet and has a scoped Send connector configured. The company's public DNS zone contains one MX record. You need to ensure that if an Internet link becomes unavailable in one data center, email messages destined to external recipients can 400-101 exam be routed through the other data center. What should you do? A. Create an MX record in the internal DNS zone B. B. Clear the Scoped Send Connector check box C. Create a Receive connector in each data center. D. Clear the Proxy through Client Access server check box Correct Answer: AQUESTION 4 Your network contains a single Active Directory forest. The forest contains two sites named Site1 and Site2. You have an Exchange Server 2016 organization. The organization contains two servers in each site. You have a database availability group (DAG) that spans both sites. The file share witness is in Site1. If a power failure occurs at Site1, you plan to mount the databases in Site2. When the power is restored in Site1, you Cisco CCNP Security 300-207 exam SITCS need to prevent the databases from mounting in Site1. What should you do? A. Disable AutoReseed for the DAG. B. Implement an alternate file share witness. C. Configure Datacenter Activation Coordination (DAC) mode. D. Force a rediscovery of the EX200 exam network when the power is restored. Correct Answer: C QUESTION 5 A new company has the following: Two offices that connect to each other by using a low-latency WAN link In each office, a data center that is configured as a separate subnet Five hundred users in each office You plan to deploy Exchange Server 2016 to the network. You need to recommend which Active Directory deployment to use to support the Exchange Server 2016 deployment What is the best recommendation to achieve the goal? A. Deploy two forests that each contains one site and one site link. Deploy two domain controllers to each forest. In each forest configure one domain controller as a global catalog server B. Deploy one forest that contains one site and one site link. Deploy four domain controllers. Configure all of the domain controllers as global catalog servers. C. Deploy one forest that contains two sites and two site links. Deploy two domain controllers to each site in each site, configure one domain controller as a global catalog server D. Deploy one forest that contains two sites and one site link. Deploy two domain controllers to each site. Configure both domain controllers as global catalog servers Correct Answer: C QUESTION 6 How is the IBM Content Template Catalog delivered for installation? A. as an EXE file B. as a ZIP file of XML files C. as a Web Appli cati on Archive file D. as a Portal Application Archive file Correct Answer: D QUESTION 7 Your company has a data center. The data center contains a server that has Exchange Server 2016 and the Mailbox server role installed. Outlook 300-101 exam anywhere clients connect to the Mailbox server by using thename outlook.contoso.com. The company plans to open a second data center and to provision a database availability group (DAG) that spans both data centers. You need to ensure that Outlook Anywhere clients can connect if one of the data centers becomes unavailable. What should you add to DNS? A. one A record B. two TXT records C. two SRV records D. one MX record Correct Answer: A QUESTION 8 You have an Exchange Server 2016 EX300 exam organization. The organization contains a database availability group (DAG). You need to identify the number of transaction logs that are in replay queue. Which cmdlet should you use? A. Test-ServiceHealth B. Test-ReplicationHealth C. Get-DatabaseAvailabilityGroup D. Get-MailboxDatabaseCopyStatus Correct Answer: D QUESTION 9 All users access their email by using Microsoft Outlook 2013 From Performance Monitor, you discover that the MSExchange Database\I/O Database Reads Average Latency counter displays values that are higher than normal You need to identify the impact of the high counter values on user connections in the Exchange Server organization. What are two client connections 400-051 exam that will meet performance? A. Outlook on the web B. IMAP4 clients C. mobile devices using Exchange ActiveSync D. Outlook in Cached Exchange ModeE. Outlook in Online Mode Correct Answer: CE QUESTION 10 You work for a company named Litware, Inc. that hosts all email in Exchange Online. A user named User1 sends an email message to an Pass CISCO 300-115 exam - test questions external user User 1 discovers that the email message is delayed for two hours before being delivered. The external user sends you the message header of the delayed message You need to identify which host in the message path is responsible for the delivery delay. What should you do? A. Review the contents of the protocol logs. B. Search the message tracking logs. C. Search the delivery reports 200-355 exam for the message D. Review the contents of the application log E. Input the message header to the Exchange Remote Connectivity Analyzer Correct Answer: E QUESTION 11 You have an Exchange Server 2016 organization. The organization contains three Mailbox servers. The servers are configured as shown in the following table You have distribution group named Group1. Group1 contains three members. The members are configured as shown in the following table. You discover that when User1 sends email messages to Group1, all of the messages are delivered to EX02 first. You need to identify why the email messages sent to Group1 are sent to EX02 instead. What should you identify? A. EX02 is configured as an expansion server. B. The arbitration mailbox is hosted 300-320 exam on EX02.C. Site2 has universal group membership caching enabled. D. Site2 is configured as a hub site. Correct Answer: A
Home / Saúde / Síndrome de McCune-Albright

Síndrome de McCune-Albright

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Definição

A condição de McCune-Albright é uma doença extremamente rara que afeta os ossos, a pele e o sistema endócrino.

Crianças com a síndrome de McCune-Albright enfraqueceram os ossos que se quebram facilmente.

O distúrbio está presente no nascimento, mas os sintomas podem não aparecer até mais tarde na infância.

A síndrome de McCune-Albright afeta igualmente meninos e meninas.

O que é

A síndrome de McCune-Albright é uma doença com base genética.

Esta condição de saúde pode afetar a coloração da pele, bem como ter um impacto negativo no desenvolvimento adequado do tecido ósseo.

Em casos extremos, a síndrome de McCune-Albright pode desencadear o surgimento de gigantismo, uma condição na qual a estrutura esquelética cresce além do que é considerado normal.

Enquanto a doença tende a estar associada ao sexo feminino e é frequentemente citada como a razão para a ocorrência de menstruação prematura em meninas, a síndrome de McCune-Albright também pode ocorrer em homens.

Em ambos os casos, os jovens de ambos os sexos podem experimentar o aparecimento precoce de pelos no corpo, especialmente nas áreas axilar e púbica.

As meninas podem começar a desenvolver seios antes de completar dez anos de idade. Tanto meninos quanto meninas serão mais suscetíveis a ossos quebrados.

Existem vários sintomas associados à presença da síndrome de McCune-Albright. O mais comum é o aparecimento de manchas na pele.

Essas manchas geralmente têm forma irregular e tendem a aparecer em manchas nas costas, embora possam ocorrer em outras áreas. Conhecidas como manchas café-com-leite, as manchas podem causar um leve desconforto, embora raramente se tornem dolorosas.

Outros sintomas comuns da síndrome de McCune-Albright incluem o desenvolvimento de tecido cicatricial na estrutura do esqueleto.

O acúmulo de tecido extra nos ossos pode pressionar as terminações nervosas e causar muita dor. A pressão no sistema nervoso pode afetar a visão. Se não for tratada, a cegueira pode resultar. Da mesma forma, o crescimento ósseo anormal pode criar problemas com a audição, até e incluindo a surdez completa.

Atualmente, não há cura conhecida para a síndrome de McCune-Albright.

Os tratamentos são geralmente direcionados para gerenciar as condições criadas pela doença. Por exemplo, o uso de drogas que se destinam a corrigir um desequilíbrio de estrogênio no organismo, como a testolactona, muitas vezes provam ser eficazes para impedir o desenvolvimento prematuro.

A síndrome de McCune-Albright não é uma doença transmissível. Não parece haver qualquer prova de que a doença é herdada de gerações anteriores na linhagem familiar.

A maioria dos profissionais médicos concorda que o desenvolvimento da doença é um evento esporádico que ocorre no útero quando ocorre uma mutação do gene GNAS1.

A mutação estará presente em várias células do corpo e desencadeará vários sintomas da doença.

Nem todas as pessoas que têm Síndrome de McCune-Albright experimentarão uma gama completa de sintomas. Mesmo dentro do contexto dos sintomas que se manifestam, a gravidade pode cair entre leve a pronunciada. Os médicos muitas vezes são capazes de trabalhar com o paciente e identificar maneiras de gerenciar a condição para que o doente tenha uma excelente chance de viver uma vida normal e saudável.

Descrição

A síndrome de McCune-Albright é um distúrbio que afeta os ossos, a pele e vários tecidos produtores de hormônios (endócrinos).

Pessoas com a síndrome de McCune-Albright desenvolvem áreas de tecido fibroso (fibroso) anormal nos ossos, uma condição denominada displasia fibrosa poliostótica. Poliostico significa que as e as anormais (less) podem ocorrer em muitos ossos; muitas vezes eles estão confinados a um lado do corpo. A substituição do osso por tecido fibroso pode levar a fraturas, crescimento irregular e deformidade.

Quando ocorrem lesões nos ossos do crânio e da mandíbula, isso pode resultar em um crescimento desigual (assimétrico) da face.

A assimetria também pode ocorrer nos ossos longos; crescimento irregular dos ossos da perna pode causar claudicação. Curvatura anormal da coluna vertebral (escoliose) também pode ocorrer.

Lesões ósseas podem se tornar cancerosas, mas isso acontece em menos de 1% das pessoas com a síndrome de McCune-Albright.

Além de anormalidades ósseas, os indivíduos afetados geralmente apresentam manchas castanhas claras chamadas manchas café-com-leite, que podem estar presentes desde o nascimento.

As bordas irregulares das manchas café-com-leite na síndrome de McCune-Albright são frequentemente comparadas a um mapa da costa do Maine. Por outro lado, manchas café-com-leite em outros distúrbios têm bordas lisas, que são comparadas com a costa da Califórnia. Assim como as lesões ósseas, as manchas café-com-leite na síndrome de McCune-Albright podem aparecer em apenas um lado do corpo.

Meninas com a síndrome de McCune-Albright podem atingir a puberdade precocemente. Essas meninas costumam ter sangramento menstrual aos 2 anos.

Acredita-se que esse início precoce da menstruação seja causado pelo excesso de estrogênio, um hormônio sexual feminino, produzido por cistos que se desenvolvem em um dos ovários.

Menos comumente, os meninos com a síndrome de McCune-Albright também podem experimentar a puberdade precoce.

Outros problemas endócrinos também podem ocorrer em pessoas com a síndrome de McCune-Albright.

A glândula tireóide, um órgão em forma de borboleta na base do pescoço, pode se tornar alargada (dos indivíduos afetados produzem quantidades excessivas de hormônio tireoidiano (hipertireoidismo), uma condição chamada bócio) ou desenvolver massas chamadas nódulos. Cerca de 50% resultando em ritmo cardíaco acelerado(uma estrutura na base do cérebro que produz vários hormônios), pressão alta, perda de peso, tremores, sudorese e outros sintomas.

A glândula pituitária pode produzir muito hormônio do crescimento uma condição caracterizada por grandes mãos e pés.

O excesso de hormônio do crescimento pode resultar em acromegalia, artrite e características faciais distintas que são frequentemente descritas como O excesso de secreção do hormônio de crescimento também pode levar ao aumento da expansão da displasia fibrosa nos ossos, “grosseiras”, mais visivelmente no crânio um excesso do hormônio cortisol produzido pelas glândulas supra-renais.

Raramente, os indivíduos afetados desenvolvem a síndrome de Cushing, que são pequenas glândulas localizadas no topo de cada rim diminui o crescimento em crianças, pele frágil, fadiga e outros problemas de saúde.

A síndrome de Cushing causa ganho de peso na face e na parte superior do corpo.

Em pessoas com a síndrome de McCune-Albright, a síndrome de Cushing ocorre apenas antes dos 2 anos de idade.

Problemas em outros órgãos e sistemas, tais como tumores gastrintestinais não benignos (benignos) chamados pólipos e outras anormalidades, também podem ocorrer na síndrome de McCune-Albright.

Fonte: Equipe Portal São Francisco

 

 

 

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